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Fructose Intolerance (Intolerance, Fructose)

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An autosomal recessive fructose metabolism disorder due to deficient fructose-1-phosphate aldolase (EC 2.1.2.13) activity, resulting in accumulation of fructose-1-phosphate. The accumulated fructose-1-phosphate inhibits glycogenolysis and gluconeogenesis, causing severe hypoglycemia following ingestion of fructose. Prolonged fructose ingestion in infants leads ultimately to hepatic failure and death. Patients develop a strong distaste for sweet food, and avoid a chronic course of the disease by remaining on a fructose- and sucrose-free diet.

Also Known As:

Intolerance, Fructose; Fructose Aldolase B Deficiency; Fructose-1,6-Biphosphate Aldolase Deficiency; Fructose-1-Phosphate Aldolase Deficiency; Hereditary Fructose Intolerance; Aldolase Deficiencies, Fructose-1,6-Biphosphate; Aldolase Deficiencies, Fructose-1-Phosphate; Aldolase Deficiency, Fructose-1,6-Biphosphate; Aldolase Deficiency, Fructose-1-Phosphate; Deficiencies, Fructose-1,6-Biphosphate Aldolase; Deficiencies, Fructose-1-Phosphate Aldolase; Deficiency, Fructose-1,6-Biphosphate Aldolase; Deficiency, Fructose-1-Phosphate Aldolase; Fructose 1 Phosphate Aldolase Deficiency; Fructose 1,6 Biphosphate Aldolase Deficiency; Fructose Intolerance, Hereditary; Fructose Intolerances; Fructose Intolerances, Hereditary; Fructose-1,6-Biphosphate Aldolase Deficiencies; Fructose-1-Phosphate Aldolase Deficiencies; Hereditary Fructose Intolerances; Intolerance, Hereditary Fructose; Intolerances, Fructose; Intolerances, Hereditary Fructose

Networked: 195 relevant articles (1 outcomes, 14 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Galactosemias (Galactosemia)
2.	Abdominal Pain (Pain, Abdominal)
3.	Congenital Disorders of Glycosylation
4.	Parasitic Diseases (Parasitic Disease)
5.	Renal Insufficiency (Renal Failure)

Experts

1.	Tolan, Dean R: 5 articles (03/2015 - 12/2002)
2.	Salvatore, Francesco: 3 articles (12/2010 - 11/2002)
3.	Vitagliano, Luigi: 3 articles (12/2010 - 11/2002)
4.	Zagari, Adriana: 3 articles (12/2010 - 11/2002)
5.	Esposito, Gabriella: 3 articles (12/2010 - 11/2002)
6.	Mavridou, Irene: 2 articles (10/2012 - 03/2009)
7.	Moraitou, Marina: 2 articles (10/2012 - 03/2009)
8.	Pollak, Agnieszka: 2 articles (10/2012 - 04/2006)
9.	Dimitriou, Evangelia: 2 articles (10/2012 - 03/2009)
10.	Michelakakis, Helen: 2 articles (10/2012 - 03/2009)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Fructose Intolerance:

1.	CarbohydratesIBA 01/01/1999 - "Offending carbohydrates are eliminated from the diet of patients with galactosemia and hereditary fructose intolerance. " 05/01/1998 - "Hereditary fructose intolerance (HFI, OMIM 22960), caused by catalytic deficiency of aldolase B (fructose-1,6-bisphosphate aldolase, EC 4.1.2.13), is a recessively inherited condition in which affected homozygotes develop hypoglycaemic and severe abdominal symptoms after taking foods containing fructose and cognate sugars. " 11/20/1994 - "Hereditary fructose intolerance is a human autosomal recessive disease caused by a deficiency of aldolase B that results in an inability to metabolize fructose and related sugars. " 06/17/1988 - "Hereditary fructose intolerance (HFI) is a human autosomal recessive disease caused by a deficiency of aldolase B that results in an inability to metabolize fructose and related sugars. " 06/24/1983 - "This case recalls the fact that fructose, sorbitol or invert sugars should not be added to infusion solutions as they may be toxic for healthy persons and imply a lethal risk for patients with undiagnosed hereditary fructose intolerance, even well beyond the baby and infant period."
2.	Fructose-Bisphosphate Aldolase (Aldolase)IBA 01/26/1995 - "Thus recombinant aldolase B is a valid model for the native enzyme and can be used to study mutations that cause hereditary fructose intolerance or others designed in the active site. " 10/01/1982 - "Molecular studies of liver aldolase B in hereditary fructose intolerance using blotting and immunological techniques." 03/01/2015 - "Aldolase-B knockout in mice phenocopies hereditary fructose intolerance in humans." 12/01/2010 - "Mutations in the promoter region of the aldolase B gene that cause hereditary fructose intolerance." 04/01/2010 - "This review gives a general overview of the features of hereditary fructose intolerance, then concentrates on the biochemistry of the AP variant (Ala149Pro variant of aldolase B) and molecular pathological consequences of mutation of the aldolase B gene."
3.	FructoseIBA 09/01/2012 - "On the other hand, in a double-blind randomized study, a dose of 25 g fructose was suggested as the most appropriate for testing individuals with suspected fructose malabsorption, whereas symptom reliability to diagnose fructose intolerance was inaccurate. " 06/01/1987 - "A study of treatment practices of pediatric centers managing hereditary fructose intolerance and a review of recent literature on this subject were undertaken in an attempt to establish the degree of dietary liberalization allowable with age and the acceptability of foods containing trace amounts of fructose. " 09/01/2015 - "Our findings are thus relevant for clinical practice, highlighting that fructose breath test is a helpful, noninvasive method by: demonstrating fructose intolerance in patients with SSc; and identifying the group of SSc patients with fructose intolerance who may benefit from low-fructose diet. " 07/01/2015 - "The effect of a tailored fructose-restricted diet on gastrointestinal complaints was assessed in patients with fructose intolerance. " 01/01/2015 - "Hereditary fructose intolerance (HFI) is a difficult-to-confirm diagnosis, requiring either invasive liver biopsy-enzyme assay or potentially hazardous fructose challenge test or expensive molecular genetic analysis. "
4.	Glucose (Dextrose)FDA LinkGeneric 01/01/1977 - "Studies of glucose turnover and renal function in an unusual case of hereditary fructose intolerance." 09/01/1981 - "The time course of D-glucose uptake, studied in two cell lines from patients with hereditary fructose intolerance, was significantly higher than for the control lines." 01/01/1987 - "The utilization of fructose and glucose by fibroblast cultures obtained from patients with hereditary fructose intolerance (HFI) was studied in comparison with fibroblast controls. " 01/01/1987 - "Comparative use of glucose and fructose in cultured fibroblasts from patients with hereditary fructose intolerance." 06/01/1987 - "The following types of carbohydrate intolerance are discussed as a risk in infusion therapy: Hereditary fructose intolerance, fructose-1,6-biphosphatase deficiency, impairment of glucose utilization during the post-aggression syndrome and/or in latent or overt diabetes mellitus. "
5.	Uric Acid (Urate)IBA 11/01/1990 - "31P magnetic resonance spectroscopy studies of children with hereditary fructose intolerance revealed a readily detectable rise in phosphomonoesters with a marked fall in inorganic phosphate in their liver in vivo and a rise in serum urate in response to very low doses of oral fructose. " 10/01/2000 - "Similar to what is seen in patients with hereditary fructose intolerance, this may increase purine nucleotide degradation and thereby increase uric acid production. " 10/01/1975 - "In two patients with hereditary fructose intolerance (HFI) the peak blood uric acid levels were 12.1 and 7.6 mg/100 ml, respectively, after fructose. " 08/01/1987 - "A marked hypoglycemia and lactacidosis, an increase in uric acid, an acute liver failure with breakdown of excretory and synthetic function and disorder of hemostasis were typical of fructose/sorbitol infusion in hereditary fructose intolerance. "
6.	FructosuriaIBA 01/01/1972 - "Studies on essential fructosuria and on hereditary fructose intolerance." 01/01/1964 - "[ESSENTIAL FRUCTOSURIA AND FRUCTOSE INTOLERANCE]." 06/01/1968 - "IV. Enzyme deficiencies: essential fructosuria, fructose intolerance, and glycogen-storage disease." 05/11/1965 - "[Metabolism of fructose, essential fructosuria and hereditary fructose intolerance]."
7.	HydrogenIBA 01/01/2003 - "A 13-year-old boy is reported with chronic unspecific abdominal pain and growth retardation and so far misdiagnosed hereditary fructose intolerance (HFI), who developed life-threatening adverse effects during the fructose breath hydrogen test. " 04/01/2014 - "The purpose of the present study was to ascertain whether pediatric patients with chronic abdominal pain had concurrent fructose intolerance as determined by a standardized dose breath hydrogen test (BHT), and whether symptoms would improve with a low-fructose diet. "
8.	Aldehyde-LyasesIBA 01/01/1974 - "Studies on liver aldolases in hereditary fructose intolerance." 09/15/2000 - "Our results show that the Arg(303)-->Gln substitution is a novel mutation causing hereditary fructose intolerance and provide a functional demonstration that Arg(303), a conserved residue in all vertebrate aldolases, has a dominant role in substrate binding during enzyme catalysis."
9.	Immunoglobulins (Immunoglobulin)IBA 09/01/2008 - "This study applied yolk immunoglobulins immunoaffinity separation and MALDI-TOF MS for clinical proteomics of congenital disorders of glycosylation (CDG) and secondary glycosylation disorders [galactosemia and hereditary fructose intolerance (HFI)]. "
10.	Genetic Markers (Genetic Marker)IBA 10/01/1987 - "This polymorphism may be used as a genetic marker to study individuals affected by hereditary fructose intolerance."

Therapies and Procedures

1.	Diet Therapy (Therapy, Diet) 06/01/1970 - "[Histological study of the liver in congenital fructose intolerance under diet therapy]."
2.	Nutrition Therapy (Medical Nutrition Therapy) 07/01/2010 - "Is medical nutrition therapy (MNT) the same for hereditary vs dietary fructose intolerance?"
3.	Protein-Restricted Diet (Diet, Protein Restricted) 02/01/2002 - "In patients with galactosaemia, hereditary fructose intolerance or tyrosinaemia type I, the presentation is dominated by a liver failure requiring galactose and fructose exclusion associated with a low-protein diet. "
4.	Minor Surgical Procedures (Minor Surgery) 01/01/1993 - "The occurrence of fatal hepatorenal failure in a 16-year-old girl, who had received infusions of fructose and sorbitol during minor surgery, led us to suspect that she had suffered from hereditary fructose intolerance. "
5.	Nephrectomy 08/01/1987 - "In connection with nephrectomy fatal liver and renal failure occurred after infusion of 50 g sorbitol in a 28 years old patient with undiagnosed fructose intolerance. "